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Bone Marrow Failure
Clinical Medicine II
Question | Answer |
---|---|
Three types of malignant neoplasms | leukemia, lymphoma, multiple myeloma |
Bone marrow failure | aplastic anemia, myelodysplastic syndrome |
Myeloproliferative conditions | polycythemia vera |
Tumors of hematopoietic and lymphoid tissue | malignant neoplasms |
4 classifications of malignant neoplasms | morphologic, immunophenotypic, genetic, clinical features |
Diff b/w acute and chronic leukemia | acute: undifferentiated, rapid, chronic: mature cells, prolonged onset |
Lymphoid cells, types leukemia, and other cancers | t and b cells, ALL, CLL, lymphoma: hodgin, NHL, Multiple myeloma |
Myeloid cells, types | granulocytes, monocytes, AML, CML |
Tumor involving lymph tissue | lymphoma |
Two most common types of leukemia in adults, peds | AML and CLL Peds: ALL |
In general what leads to leukemia | chromosomal translocations→activate oncogenes and ↓Tumor Suppressor genes |
What happens within the cell | nl cell development arrested, rapid proliferation of tumor cells→↓apoptosis so may cells, nl cells can’t form |
What are the 3 results w/I the body | anemia, thrombocytopenia, neutropenia |
Causes or etiology of leukemia | exposures: carcinogenic, radiation, chemo,benzene, hair dyes, genetic, pesticides |
What congenital d/oss predisposed for leukemia | down syndrome, congenital neutropenia, fanconi anemia, neurofibromatosis |
D/O of blood and blood forming organs | leukemia |
Fatigue, weakness doe, dizziness | anemia |
Infections/fever, oral throat, respiratory, uti, skin infx | neutropenia |
Bleeding-skin, bums, mucous membranes, GI, GU, brusing | thrombocytopenia |
Reasons for bone pain, LUQ pain | medullary bone marrow cancer, splenomegaly |
What is a painless mass | lymphadenopathy |
What are B symptoms | generalized wt loss, fever, drenching night sweats |
Signs of leukemia | anemia, neutropenia, thromobycytopenia: petchiae, eccymoses, spleen, liver enlargement and lymphadenopathy |
How do we Dx leukemia | CBC: anemia, neutropenia, thrombocytopenia Peripheral smear: could see proliferation specific cells, dx lineage. bone marrow aspirate |
Diagnosis for AML, CML, ALL, CLL | auer rods, Philidelphia chromosome, CLL: smudge cells |
When is CML seen | middle aged |
Tx in general for leukemia | supportive therapy, chemo, HSCT, observation |
Three stages of chemo | (remission) indusction, consolidation: post remission therapy, maintenance therapy |
What should we monitor for w/ chemo | cytopenias: infx, bleeding, tumor lysis syndrome |
Most electrolytes are elevated→renal failure | tumor lysis syndrome |
3 ways to use HSCT | bone marrow, peripheral blood, umbilical core blood |
What is autologous and allogenic mean | autologous: self, allogenic: someone else: problem: graph vs. host dz |
3 phases of CML | initial: chronic, Accerlerated phase (transitional), blastic phase: similar to acute |
What is associated w/ Philadelphia chromosome | CML 9 and 22 cross over aka BCR-ABL gene |
Two types of lymphoid neoplasms | lymphocytic keukemia ALL, CLL and lymphoma: Hodking and Non Hodgkin lymphoma |
What are proliferation of lymphoid cell lines in the bone marrar and peripheral blood | lymphocytic leukemia |
Wjere does lymphocytic leukemia infiltrate | into lymph organs, such as lymph nodes, spleen, liver: can present w/ mas |
Two types of lymphoid leukemias | ALL: 85%B cell 15% T cell and CLL: B cell 90% both may/may not have a lesion |
Symtoms include for ALL | mild b symptoms, bone pain, HSM , LAD, CNS, mediastinal mass |
Symptoms included for CLL | painless LAD, B symptoms, HSM, skin |
What is lymphoma | a lymphoid tissue mass, two main types: Hodgkin and non Hodgkin (all others) |
What are reed-sternberg cells associated with | hodgkin lymphoma |
What is HL associated with | EBV, immunosuppression, familial link: exposures and genetics |
Clinical signs of HL | nontender lymphadenopthay: cervical and supraclavicular, Mediastinal mass: SOB, B sxs, pruritus, splenomegaly |
Dx of HL | CBC often nl, lymph node biopsy ++ for reed-sternberg cells |
HL tx | radiation alone, chemo alone, combined often very high survival |
Main dx of HL | lymph node progression in order, and reed-sternberg cells, involves B cells |
Malignant solid tumors of lymph tissue | can begin in any region of lymphatic sxs: Non-hodgkins lymphoma |
Types of NHL | small lymphocytic lymphoma, mantle cell lymphoma, burkitt lymphoma, large B;cell lymphoma, involves many types of cells |
When do you biopsy a lymph node | >2cm, persistence >4-6weeks, progressive increase in size |
NHL presentation | lymphadenopathy, HSM, B sxs, fever unknown origin |
What will t-cell involvement present as | skin rash and pruritis |
Sxs associated w/ extranodal tissues w/ NHL | pain in chest, abd, bone, GI: N/V anorexia abd fullness, CNS lethargy FND |
Dx of NHL | lymph node/tissue biopsy, bone marrow biopsy, morphology and phenotype, CBCs, chemistries, LFTs LDH Imaging |
When do you think NHL | painless masses, or abnl size etc |
NHL tx | radiation chemo bio therapy watchful waiting |
Monoclonal abs given to carry drugs, toxins or radioactive material directly to cancer cells | biology therapy |
When is NHL prognosis worse | >60yo, increase LDH, stage III or IV, involved extranodal dz sites >1, ↓ fxn capacity |
Tumors of differentiated B cells | plasma cell neoplasms, arise in bone marrow |
Two types of plasma cell neoplasm | PCM: single plasmacytoma, MM: multiple myeloma lesions |
Causes of MM | rad, benzene, herbicides, insecticides, old age, |
Sxs for multiple myeloma (CRAB) | calcium (elevation), renal failure, anemia, bone lesions |
Why is calcium elevated | extensive skeletal destruction d/t osteoclast activation →↑Ca+ in blood |
Clinical signs of MM | ↑Ca+ and bone pain d/t destruction of bone, can see osteoporosis, frxs, especially in spine ribs and w/movement |
What is the anemia in MM | bone marrow suppression, ↓ RBC d/t infiltration of tumor cells Signs: fatigue SOB, weakness |
How do we dx MM w/ UA | casts (sediments), and bence jones proteremia |
Fragments of abs in the urine | bence-jones proteiremeia |
Stacked blood cells d/t excess proteins in blood | Rouleaux formation |
Different methods for MM dx | UA, blood smear, monoclonarl proteins in serum/urine, bone marrow biopsy:clonal plasma cells, radiographs |
Tests to identify MM | XR for osteolytic lesions, CT, MRI |
TX MM | narcotics, bisphosphonates to prevent bone lesion, erythropoietin or blood trfx, abx/vaccines, metabolic |
Is MM curable | no,no tx: 6m death w/ tx: 2-5yrs |
Causes of bone marrow failure | bone marrow replacement: tumor, fibrosis, Congenital: fanconi anemia, acquired: aplastic and myelodysplasia |
Diminished or absent hematopoietic precurses in the bone marrow | aplastic anemia dx: pancytopenia sxs |
Causes of aplastic anemia | many drugs, chemicals,viruses, radiation, idiopathic |
Patho of AA | autoimmune, immune suppression |
Clinical signs of AA infections, mucosal hemorrhage: thrombocytopenia, fatigue, cardiopulmonary compromise: anemia, pallor, but NL liver spleen lymph nodes | |
Dx AA | pancytopenia, normocytic cells, but reduced number no abnormal cells anywhere in any bx or smears |
Tx AA | withdraw causative agents, supportive care, HPCT, immunosuppression |
Problem w/ AA | can progress to AML, MDS, and PNH |
Ineffective blood cell production | myelodysplastic syndrome: cytopenias, dysmorphic, |
Risks of MDS | >65yo, radiation, benzene chemo etc |
Dx of MDS | CBC: anemia, smear: could be normo, marcrocytic, but bone marrow aspirate/biopsy will be hypercelluar w/ abnl cells |
Prognosis of MDS | 50% 3yr, many will progress to AML will vary |
Myeloproliferative d/o | polycythemia vera: huge number of RBC’s |
Sxs of PCV | vertigo, tinnitus, HA, visual disteracnes, HTN, thrombosis, TIA splenomegaly, excoriations, GI distress (all d/t ↑RBC) |
Dx of PCV | CBC Erythrocytosis +/- leukocytosis, hypercellularity on bone marrow aspirate, low erythropoietin, O2 low |
Tx of PCV | phlebotomy and aspirin |
Untreated PCV complications | thrombosis, AML progression, can do well for years |
Cell number and appearance/fxn of cells in AA, Myeloid, polycythemia vera | AA: pancytopenia, no fxn, Myleoid: Anemia/nl Fxn: abnl PV: erythrocytosis Fxn nl |